title: Extranodal rosai-dorfman disease associated with increased numbers of immunoglobulin g4 plasma cells involving the colon: case report with literature review.
abstract: A 49-year-old woman presented with fever, weight loss, night sweats, hematochezia, and acid reflux symptoms. Two large, firm cecal lesions were seen at colonoscopy, but multiple biopsies were inconclusive. The patient underwent a right hemicolectomy for a clinical diagnosis of colon cancer. Noncaseating granulomatous inflammation with background lymphocytes, plasma cells, and histiocytes exhibiting emperipolesis were identified. With these histologic features and immunoreactivity for S-100 protein and CD68, a diagnosis of Rosai-Dorfman disease was rendered. Other areas had storiform fibrosis admixed with numerous immunoglobulin G4 (IgG4)-positive plasma cells. Although a few preliminary reports have noted an increased number of IgG4-positive plasma cells in Rosai-Dorfman disease, the relationship between these 2 conditions is unclear. To our knowledge, this is the first case report of a possible association of colonic Rosai-Dorfman disease with an increased number of IgG4-positive plasma cells. Reviews of colonic Rosai-Dorfman disease and IgG4-related sclerosis are presented to heighten awareness of this rare presentation.
authors.affiliation: Departments of Pathology and Genomic Medicine, The Methodist Hospital, Weill Medical College of Cornell University, Houston, Texas 77030, USA. aayala@tmhs.org
authors.forename: Daniel B
authors.initials: DB
authors.lastname: Wimmer
authors.forename: Jae Y
authors.initials: JY
authors.lastname: Ro
authors.forename: Annisa
authors.initials: A
authors.lastname: Lewis
authors.forename: Mary R
authors.initials: MR
authors.lastname: Schwartz
authors.forename: Richard
authors.initials: R
authors.lastname: Caplan
authors.forename: Peter
authors.initials: P
authors.lastname: Schwarz
authors.forename: Alberto G
authors.initials: AG
authors.lastname: Ayala
mesh_terms: D015703:Antigens, CD / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000378:metabolism; D001327:Autoimmune Diseases / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D015415:Biomarkers / Q000378:metabolism; D003082:Colectomy; D003108:Colonic Diseases / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D005260:Female; D015618:Histiocytosis, Sinus / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood* / Q000276:immunology; D008198:Lymph Nodes / Q000473:pathology; D008875:Middle Aged; D010950:Plasma Cells / Q000378:metabolism / Q000473:pathology*; D009418:S100 Proteins / Q000378:metabolism; D012598:Sclerosis / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D000097382:CD68 Molecule
chemical_list: D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D015415:Biomarkers; D007074:Immunoglobulin G; D009418:S100 Proteins; C067980:CD68 antigen, human; D000097382:CD68 Molecule